Noah's Story

Welcome to Noah's story. I've decided to write this blog to help others who are facing Pierre Robin Sequence and its obstacles. Along our journey the web has been our source of information. Everything from scientific explanations, to stories like ours, to images. We have found multiple sources that explain what Pierre Robin is and even some touching stories of families who have travelled along this path. Still many of these resources didn't chronicle the child's developmental milestones or growth. Therefore, I decided to make it  my mission to share with you our experience with PRS in hopes that our story can help you. While this journey has not been an easy one, we've survived and conquered PRS and it's challenges with the help of our Lord Jesus Christ. I can't share our story without sharing all about HIS grace, love, and strength in the most difficult experience I've had as a mother. Christ has been our center and our strong hold in the most desperate and frightening scenarios and we praise HIM for the work he has done in us and in our Noah.

As a Foreword....
My husband and I have been married for 8 years as of 2012. We welcomed our first child in January 2009, Caleb. Caleb has been our "perfect gift from above" and has brought much joy to our lives and to our family as a whole. In 2011, we decided we wanted to have another. As with Caleb we quickly were expecting. Baby #2 was due two days after Caleb's 3rd birthday. My pregnancy evolved without a hitch. I gained just the right amout of weight and all my ultrasounds revealed that baby #2 was a healthy baby boy we named Noah Levi. Towards my 36 weeks I was measuring larger than I should have so an ultrasound was done. My ultrasound only revealed that my amniotic fluids were low so we had a stress test. That resulted normal. I had been experiencing contractions off and on for weeks and was already 2cm dilated. The following week I had yet another ultrasound and stress test. All seemed fine though the ultrasound technician asked multiple times if I had had a genetics test, which we hadn't. The following evening my contractions became stronger and consistent.  By 11pm on December 29th we were checking in to the hospital. Labor went as expected... strong contractions, epidural heaven, and severe back labor. By 5:45 we started pushing and Noah Levi was born at 5:54am. He was a crier. At 37 weeks, on December 30th,  he weighed in at 7.2lbs and 18 1/2 inches long. We thought he was beautiful and didn't think anything was wrong. But there was....

 Breathing on his own...before mandibular distraction...proving to us all that he was a fighter and the LORD was with him.

Pierre Robin Sequence/Syndrome

During Noah's newborn evaluation, the nurse noticed that his jaw was pushed far back and that he had a cleft palate. They sent him off to the NICU to be evaluated and observed. Dad followed him along.

Next time I saw Noah I held him for a few minutes as tears flowed down. He had tubes and wires hanging everywhere. I cried at the sight of him but I had hope that everything would be okay. How could it not? I expected nothing but good news in the next hours and expected to take him home in two days. Well that didn't happen. Soon we were told his air way obstruction had caused his lung to collapse, Drs were successful in inflating it again. By 3pm the Dr told us Noah would be transferred over to the Children's Hospital of Philadelphia for specialized care. They told us the name of the condition but explained nothing further nor what it meant. He had Pierre Robin Sequence/Syndrome. A condition in which the jaw was underdeveloped. In the womb it didn't have a chance to pop forward during development which resulted in a cleft palate. A condition that made the two major functions of life: breathing and eating difficult.  He was in the NICU for the rest of the day.

Family came to visit and meet Noah. No one knew exactly what to expect or what was happening but when they saw him, they cried as did I. By 9pm Noah was transported and Daddy went with him. I was left behind. Still recovering...with a broken heart...and alone. Still clueless to what lied ahead.

First Month...Mandibular Distraction...Hopefulness

Less than 24 hours after Noah's birth he was transferred over to the local Children's Hospital. Immediately he was surrounded by a Pediatric team that included ENT, Plastics, and a Genetics Doctor. The renown Genetic Dr evaluated Noah and gave her opinion that this PRS case was not genetic as Noah's features did not resemble any syndrome however a genetics test would still be given and would take 4-6 weeks. During the next 24 hours we were educated on PRS. We learned the possibilities that PRS was linked to other syndromes and health concerns.As we continued to be educated and practically frightened with all the worse scenarios, our little Noah had a breathing tube and a feeding tube in place. The Drs discussed the various procedures that were performed for PRS. They seemed to lean towards Mandibular Distraction. .....

The next four weeks they evaluated Noah for Stickler's syndrome, checked him for a heart murmur, checked his hearing, evaluated his lungs and heart, had physical therapy, a speech therapist, and even circumcised him. All his tests would return normal. Before leaving we got the results of the genetics test that concluded that PRS was not genetically linked. This was a relief. Any genetic mutation put Noah at a higher risk of having complications of the heart or brain.  Doctors couldn't tells us exactly how this happen; they could only suggest that sometimes PRS is a result of fetal crowding.

We didn't know what to expect and how long Noah would be admitted. The first week they had him intubated and he barely was fed. I couldn't nurse him and because of his condition he wasn't given a bottle so I pumped breastmilk around the clock to stimulate production. I pumped every 2 hrs.They fed him very little via a feeding tube. By the end of that week they took him off the breathing tubes and tried letting him breath on his own. Noah did well and didn't need anything but a nasal cannula but required specific positioning. We hope this meant that he didn't need surgery but 3 days later the Drs confirmed that mandibular distraction was the best option.

On January 12th Noah underwent mandibular distraction. Thanks to the LORD surgery went well but Noah was intubated once again and spent about 4 days under sedation for pain management. He looked like a 4 month old baby due to the swelling on his face. When I spoke to him he would squeeze my finger and his eyes would break my heart. After a few days the the distraction began.
By week 3 Noah was up with little pain management. He now cried a whole lot. Looking back I truly believe he was hungry and suffering from acid reflux. We started bottle feeding with a special Habermann nipple for cleft palate babies. He did well but feeding took a long time and a lot of energy. He never finished his 70mls. He took 5-10 and the rest was via feeding tube. A speech therapist came daily to help.

By week 4 nothing but feeding was keeping Noah at the hospital. We were ready to stop our daily, full day trips to the hospital. Every day I prayed Noah would increase his feeds. And he did, slowly. Thankfully, a very special nurse urged  that we try another cleft palate nipple, the pigeon nipple, with him. The speech therapist wasn't very hopeful but as soon as we did Noah took all if not 95% of his feed. Within 3 days and at his 1 month mark Noah was sent home. 
He came home still with a feeding tube and we distracted for about 4 more days but there was nothing like having our baby home finally.

That month at the hospital, despite the wonderful care he received, really shrunk our little guy. He gained only 1lb more and grew only 1/2 inch.

Swollen after Mandibular Distraction...

Second Month...Difficult

Bringing Noah home was more of a nightmare than all. Yes it was wonderful to have him home but the challenges, stress, and overwhelming anxieties made for a very difficult situation. It was a situation that hurt to be in and that I hated to be in.
The first week at home was crazy. Having to feed Noah was a challenge. We still battled with the bottle and the feeding tube. It was a challenge to deal with the feeding extravaganza. Let's not forget I was also pumping breast milk around the clock to help my milk production. Add the immense crying Noah did. Mostly because he was hungry, tired, and in pain. It was not fun. By the end of the first week Noah managed to pull his feeding tube out. Although we were trained on how to insert it back in, we could not bare having to do so. And so we took a desperate measure and cut his nipple to make the opening bigger and viola! No more feeding tubes...more ounces...more satisfaction...a little less more feeding tubes! Praise the LORD!
We conquered one thing but feeding him was still a challenge. We had weekly weight checks and started him on some acid reflux meds. His pediatrician stressed he needed to drink more milk and we tried but acid reflux was not kind.
Slowly things were slightly better but Noah wasn't as easy as pie. By the end of the second month he weighed ten pounds and was doing better. He still cried a lot and well sleeping wasn't his forte. Still we could see the horizon as his distractors would come off mid March.

Month 3...Disappointment

By March Noah was growing slowly and we were beginning to feel more comfortable with the situation. He had caught an infection with the distractors and we couldn't wait to have the final surgery. A week before the surgery date we were disappointed to find that Noah's chin had regressed. Though he could eat and breath without any concerns...he physically didn't look that well.  And so his Plastic Dr had us start the process again. We started distracting for two weeks and needed to wait 6 weeks before they could come off...a new surgery date was set for late May.

In the next month Noah battled another infection and lots of bandaids and wound cleans. I still remember his cries and discomfort. He continued to grow and eat but when the acid reflux med seemed to stop working I bought Colic Calm and gave it to him. That did the more acid reflux discomfort and Noah began to really enjoy meal time.

Our faith could have been shaken after the mechanical malfunction but this was yet again another opportunity for the LORD to remind us that HE was in control. And HE really was.

Fourth Month...solids

At four months old Noah was growing. He weighed 14 lbs and was about 22 inches long. He was still sporting bandaids and his distractors. We were counting down the weeks until his surgery. Despite his challenges Noah was a wild child. He was rolling from his tummy to his back and from his back to his tummy. He had even discovered how to scoot on his back.  We would lay him at the center of the room and find him against the wall. He was still feeding through the night although he now was only getting up once and every so often he would sleep through the night. The Dr also gave us the green light on solids. After much googling, I found that some cleft babies did better with solids than others. But overall most stories were positive; moms suggested that it could take baby a while to learn to move food past the cleft and down the throat instead of up the cleft. I also read about the big sneeze, a way of baby clearing up the nasal passage from the food. And so I jumped into solids with Noah and I was thrilled to see he took it so well. He had no issues and loved food. A handful of sneezes but not often at all. Noah got it quickly.

Fifith Month....Hoorraayy!

May 21st was Noah's big day. Daddy was away in a training and I had to tackle this alone. My sister came along and I was grateful for that. But Daddy was missed. Noah was turning 5 months in about 10 days and we couldn't wait to celebrate without the distractors.

Surgery Day was long. Noah drank his milk at 6am and wasn't taken back to surgery until 1pm. Brutal. But God was good. Noah wasn't cranky; he survived the prep work and fell asleep for a nap as they rolled him away. He had no idea what hit him.

Next time I saw him He was whining and groggy. He was in pain and he was getting morphine what seemed like every 5 minutes. He was so swollen he looked like he weighed 30 lbs. I stayed with him that night. He was practically sedated and didn't wake to eat till about 3am and didn't take much. The following day he was given the okay to go home if he increased his feeds. We worked on it and back home I drove with him...alone.

The first night home he slept through the night and had one dose of pain medication. His appetite wasn't back yet and I worried a whole lot. He also wore mittens as not to pull the tape over his stitches. The next couple of days he slowly started to gain his appetite. His swelling worsen the two days after surgery. He looked like a sumo wrestler. A week after surgery he was back to his routine and looked a lot better.

We praise the LORD for bringing Noah through this. Now to heal and focus on growing.
Before prep work.


 1 week after surgery.